CONTINUUM Lifelong Learning in Neurology最新文献

Objective: This article discusses the effects of myelopathy on multiple organ systems and reviews the treatment and management of some of these effects.

Latest developments: Recent advances in functional electrical stimulation, epidural spinal cord stimulation, robotics, and surgical techniques such as nerve transfer show promise in improving function in patients with myelopathy. Ongoing research in stem cell therapy and neurotherapeutic drugs may provide further therapeutic avenues in the future.

Essential points: Treatment for symptoms of spinal cord injury should be targeted toward patient goals. If nerve transfer for upper extremity function is considered, the patient should be evaluated at around 6 months from injury to assess for lower motor neuron involvement and possible time limitations of surgery. A patient with injury at or above the T6 level is at risk for autonomic dysreflexia, a life-threatening condition that presents with elevated blood pressure and can lead to emergent hypertensive crisis. Baclofen withdrawal due to baclofen pump failure or programming errors may also be life-threatening. Proper management of symptoms may help avoid complications such as autonomic dysreflexia, renal failure, heterotopic ossification, and fractures.

Objective: This article illustrates the clinical importance, diagnosis, and management of degenerative and nondegenerative structural myelopathies. It also aims to create a diagnostic approach for the evaluation of patients with suspected degenerative myelopathies.

Latest developments: There is considerable interest in developing diagnostic methods that can assist in deciding if surgery is indicated in patients with structural myelopathy and the optimal timing for surgery. Diffusion tensor imaging has emerged as a promising imaging modality although it is not used routinely in clinical practice. Neuroprotective medications and interventions are being studied in patients with degenerative myelopathies.

Essential points: Structural myelopathies and particularly degenerative myelopathies are common disorders that are routinely encountered in clinical practice, with symptoms that frequently overlap with other neurologic disorders. The prompt diagnosis and treatment of patients are essential in achieving good functional outcomes.

Objective: Vascular injuries of the spinal cord are less common than those involving the brain; however, they can be equally devastating. This article discusses the diagnosis and management of ischemic and hemorrhagic vascular disorders of the spinal cord.

Latest developments: Clinical suspicion remains the mainstay for recognizing vascular myelopathies, yet diagnoses are often delayed and challenging in part because of their rarity and atypical manifestations. Noninvasive imaging such as CT and MRI continues to improve in spatial resolution and diagnostic precision; however, catheter-based spinal angiography remains the gold standard for defining the spinal angioarchitecture. In addition to hemorrhagic and ischemic disease, the contribution of venous dysfunction is increasingly appreciated and informs treatment strategies in conditions such as intracranial hypotension.

Essential points: Vascular disorders of the spine manifest in variable and often atypical ways, which may lead to delayed diagnosis. Increased awareness of these conditions is critical for early recognition and treatment. The goal of treatment is to minimize long-term morbidity and mortality.

Objective: This article provides an overview of genetic myelopathies, a diverse group of inherited, degenerative conditions that may be broadly categorized as motor neuron disorders, disorders of spinocerebellar degeneration, leukodystrophies, and hereditary spastic paraplegia. Clinical examples from each category are provided to illustrate the spectrum of genetic myelopathies and their distinguishing features that aid in differentiating genetic myelopathies from potentially treatable acquired causes of myelopathy.

Latest developments: Advances in genetic testing have vastly enhanced current knowledge of genetic myelopathies and the ability to diagnose and provide appropriate counseling to patients and their families. However, potential health care disparities in access to genetic testing is a topic that must be further explored. Although treatment for most of these conditions is typically supportive, there have been recent therapeutic breakthroughs in treatments for amyotrophic lateral sclerosis, spinal muscular atrophy, and Friedreich ataxia.

Essential points: Genetic myelopathies may present with chronic and progressive symptoms, a family history of similar symptoms, and involvement of other structures outside of the spinal cord. Imaging often shows spinal cord atrophy, but cord signal change is rare. Exclusion of reversible causes of myelopathy is a key step in the diagnosis. There are many different causes of genetic myelopathies, and in some cases, symptoms may overlap, which underscores the utility of genetic testing in confirming the precise underlying neurologic condition.

Abstract: The journey for a patient with a brain tumor and their loved ones can be extremely challenging due to the high burden of physical symptoms and the emotional distress caused by the diagnosis. Optimizing quality of life by addressing symptoms and reducing this emotional distress can improve treatment tolerance and outcomes and alleviate care partner distress and burden. Symptoms in patients with central nervous system (CNS) tumors can vary in onset and intensity, ranging from headaches, seizures, and focal weakness to emotional distress and cognitive dysfunction. Additionally, care partners may demonstrate distress due to the high burden of care and need appropriate support structures and access to resources to alleviate this stress. Evidence-based recommendations are unfortunately limited given the lack of high-quality research in this area, but patients living with CNS tumors and their loved ones can benefit from early and routine symptom identification and management, compassionate and transparent communication, and practical guidance for the future. These principles are part of palliative care, a field of medicine focused on alleviating suffering in patients with serious, chronic illness. Clinicians involved in the care of patients with CNS tumors must be educated in these important primary palliative care principles. This article focuses on key symptom management, strategies for high-quality communication, a discussion of advance care planning, and an overview of end-of-life care.

Objective: Progress is ongoing in understanding paraneoplastic neurologic disorders, with new syndromes and antibodies being described and more detailed evidence available to guide workup for diagnosis and treatment to improve outcomes. Many excellent reviews have summarized the molecular features of different antibodies, but this article emphasizes the clinical features of each syndrome that may help guide initial diagnosis and treatment, which often should occur before an antibody or cancer is found to confirm the diagnosis.

Latest developments: Recent findings include updated diagnostic criteria with validated sensitivity and specificity, discovery of novel antibodies, and clinical findings that increase the likelihood of an underlying paraneoplastic disorder. Suggestive syndromes that have been recently identified include faciobrachial dystonic seizures and pilomotor auras in anti-leucine-rich glioma inactivated protein 1 encephalitis, extreme delta brush on EEG in N-methyl-d-aspartate (NMDA)-receptor encephalitis, déjà vu aura in anti-glutamic acid decarboxylase 65 (GAD65) encephalitis, and sleep disturbances in several disorders. In addition, there is confirmed utility of brain positron emission tomography (PET) and CSF markers, including carcinoembryonic antigen and oligoclonal bands, as well as improved tests for the presence of leptomeningeal cancer cells in CSF. Associations of cancer immunotherapies with paraneoplastic syndromes and herpes simplex virus encephalitis (and COVID-19) with NMDA-receptor encephalitis have been described.

Essential points: All neurologists should be aware of advances regarding paraneoplastic neurologic syndromes, as patients can present with a wide variety of neurologic symptoms and earlier diagnosis and treatment can improve outcomes.